Moyamoya disease in the practice of a pediatric-infectiologist

Moyamoya disease is a rare chronic cerebrovascular disorder characterized by gradually progressive luminal stenosis of the intracranial segments of the internal carotid artery and the initial segments of the anterior and middle cerebral arteries, thus forming a network of minor vascular anastomoses. Impaired blood supply in the above vessels due to occlusion leads to the development of ischemic and hemorrhagic strokes in the respective beds, causing a variety of neurological and visual disorders.

Activation of herpesvirus infection in a 2-year-old infant provoked the onset of moyamoya disease. An abnormal posterior right eye segment (bindweed syndrome) detected by an ophthalmic examination, which is commonly concurrent with various cerebrovascular disorders, allowed cliniciansto adequately interpret mild transient neurologicalsymptoms and to rapidly diagnose moyamoya disease, by applying radiodiagnostic methods.

The treatment involved antibiotic and antiviral therapy, infusion of glucose-salt solutions and encephabol. The infant’s status improved; seizures were stopped; right muscle tone fully recovered; and right hand tone remained reduced. Revascularization of  the middle and anterior cerebral arteries were further performed. 

1. Всероссийский медицинский портал Источник: http://online-diagnos.ru/illness/d/bolezn-moya-moya. Ссылка активна на 27.02.17

2. Krishnan C., Roy A., Traboulsi E. Morning glory disk anomaly, choroidal coloboma, and congenital constrictive malformations of the internal carotid arteries (moyamoya disease). Ophthalmic Genet 2000; 21(1): 21–24.

3. Nezzar H., Mbekeani J. N., Dalens H. Morning glory syndrome with carotid and middle cerebral artery vasculopathy. Optom Vis Sci 2015; 92(12): 437–441. DOI: 10.1097/OPX.0000000000000727.

4. Papavasileiou E., Sobrin L., Papaliodis G.H. Ocular ischemic syndrome presenting as retinal vasculitis in a patient with moyamoya syndrome. Retin Cases brief Rep 2015; 9(2): 170–172. DOI: 10.1097/ICB.0000000000000129.

5. Wakai K., Tamakoshi A., Ikezaki K., Fukui M., Kawamura T., Aoki R., Kojima M., Lin Y., Ohno Y. Epidemiological features of moyamoya disease in Japan: finding from nationwide survey. Clin Neurol Neurosurg 1997; 99(Suppl. 2): 1–5.

6. Буркова К.И., Ажермачева М.Н., Алифирова В.М. Болезнь моямоя (клиническое наблюдение). Неврологический журнал 2014; 5: 38–42. [Burkova K.I., Azhermacheva M.N., Alifirova V.M. The moya-moya disease (a clinical observation). Nevrologicheskiy zhurnal 2014; 5: 38 – 42. (in Russ)]

7. Бывальцев В. А., Сузуки Й. Комбинированное лечение болезни моя-моя с использованием прямого анастомоза и реваскуляризации – опыт 225 операций. Вопросы нейрохирургии им. Н.Н. Бурденко 2007; 3: 11–16. [Byvaltsev V.A., Suzuki Y. Combined treatment for moya-moya disease using direct anastomosis and revascularization – the experience of 225 operations. Voprosy neyrokhirurgii im. N.N. Burdenko 2007; 3: 11–16. (in Russ)]

8. Скоромец А.А., Шулешова Н.В., Курилина А.П. К клинике и лечению болезни мойямойя. Вопросы нейрохирургии им. Н.Н. Бурденко 1988; 2: 28–31. [Skoromets A.A., Shuleshova N.V., Kurilina A.P. To the clinic and treatment of moya-moya. Voprosy neyrokhirurgii im. N.N. Burdenko 1988; 2: 28–31. (in Russ)]

9. Мосин И.М. Врожденные и приобретенные заболевания зрительного нерва. Руководство по клинической офтальмологии. Под ред. А.Ф. Бровкиной, Ю.С. Астахова М: Медицинское информационное агентство» 2014; 494–567. [Mosin I.M. Congenital and acquired diseases of the optic nerve. Manual of clinical ophthalmology A.F.Brovkina, Y.S. Astakhov. (eds). Moscow: «Meditsinskoye informatsionnoye agentstvo», 2014; 494–567. (in Russ)]

10. Komiyama M., Yasui T., Sakamoto H., Fujita K. Basal meningoencephalocele, anomaly of optic disc and panhypopituitarism in association with moyamoya disease. Pediatr Neurosurg 2000; 33(2): 100–104.

11. Lenhart P. D., Lambert S. R., Newman N. J., Biousse V., Atkinson D.S.Jr., Traboulsi E.I., Hutchinson A.K. Intracranial vascular anomalies in patients with morning glory disk anomaly. Am J Ophthalmol 2006; 142(4): 644–650.

12. Suzuki J., Kodama N. Moya-moya disease. A review. Stroke 1983; 14(1) 104–109.

13. Azuma N., Yamaguchi Y., Handa H., Tadokoro K., Asaka A., Kawase E., Yamada M. Mutations of the PAX6 gene detected in patients with a variety of optic nerve malformations. Am J Hum Genet 2003; 72(10): 1565–1570.

14. Ikezaki K. Rational approach to treatment of moya-moya disease in childhood. J Child Neurol 2000; 15(5): 350–356.

15. Scott R. M., Smith E. R. Moyamoya disease and moyamoya syndrome. N Engl J Med 2009; 360(12): 1226–1237. DOI: 10.1056/NEJMra0804622.